Table 1.

Genes encoding oxidative phosphorylation factors that display altered expression in MeCP2-deficient systems.

Gene	Coding genome	Protein function	Δ	Cell/tissue type	Organism	References
Mt-Nd2	mtDNA	NADH-dehydrogenase subunit 2 (complex I)	↓	Brain tissue	MeCP2-null mice	Kriaucionis et al., 2006
NDUFA 1, 2, 8, 9 NDUFB 2, 4, 6, 9, 10 NDUFC 1, 2 NDUFS 4, 5, 6 NDUFV 2	nDNA	NADH: ubiquinone oxidoreductase (complex I)	↑	Lympho-monocytes	Human (RTT patients)	Pecorelli et al., 2013
SDHB	nDNA	Succinate dehydrogenase complex, subunit B (complex II)	↑	Lympho-monocytes	Human (RTT patients)	Pecorelli et al., 2013
Uqcrc1	nDNA	Cytochrome b-c1 complex subunit1 (complex III)	↑	Brain tissue	MeCP2-null mice	Kriaucionis et al., 2006
UQCRC1	nDNA	Cytochrome b-c1 complex subunit1 (complex III)	↓	ESC derived MeCP2-null neurons	Human	Li et al., 2013
UQCRQ UQCRFS1 UQCRH	nDNA	Subunits of complex III	↑	Lympho-monocytes	Human (RTT patients)	Pecorelli et al., 2013
CO1	mtDNA	Cytochrome c oxidase subunit 1 (complex IV)	↓	Frontal and occipital cortex	Human (RTT patients)	Gibson et al., 2010
				MeCP2-null SH-SY5Y	Human	Gibson et al., 2010
				Skeletal muscles	MeCP2-null mice	Gold et al., 2014
COX14, COX6C, COX7C, 7A2, 8A	nDNA	Subunits of cytochrome c oxidase (complex IV)	↑	Lympho-monocytes	Human (RTT patients)	Pecorelli et al., 2013
ATP5A1, ATP5EP2, ATP5J2, ATP5O	nDNA	Subunits of ATP synthase (complex V)	↑	Lympho-monocytes	Human (RTT patients)	Pecorelli et al., 2013
ATPIF1	nDNA	ATPase inhibitory factor 1	↑	Lympho-monocytes	Human (RTT patients)	Pecorelli et al., 2013
ANT1	nDNA	Adenine nucleotide transporter	↑	Brain tissue, cerebellum, skeletal muscles	MeCP2-null mice	Forlani et al., 2010
CYCS	nDNA	Electron carrier cytochrome c	↑	Lympho-monocytes	Human (RTT patients)	Pecorelli et al., 2013